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Remission of Nystagmus Following Fitting Contact Lenses to an Infant with Aniridia
REMISSION O F N Y S T A G M U S F O L L O W I N G F I T T I N G C O N T A C T LENSES T O A N I N F A N T W I T H A N I R I D I A JAY M. E N O C H , P H . D . AND CHARLES E. WINDSOR, St. Louis, Missouri
Congenital aniridia has a poor visual prognosis. Glaucoma, dislocated lenses, strabismus, nystagmus, and other ocular anomalies interfere with the development of useful vision. Even if glaucoma and retinal abnormalities do not develop, uncontrolled retinal illumination, increased ocular aberrations, and decreased depth of focus due to absence of the pupillary mechanism, in some manner cause the development of nystagmus and subsequent subnormal vision. In the following case the fitting of vented scleral contact lenses with artificial irides not only eliminated the patient's photophobia, but appeared to reduce markedly his nystagmus. This case will be followed to ascertain whether these and subsequent lenses will serve to inhibit further the development of nystagmus. Grosvenor has summarized suggestions in the literature that optically-induced nystagmus shows remission following fitting of contact lenses. None of the patients reported were infants and none had aniridia. Iliff and Naquin indicated that contact lenses failed to improve vision, but gave visual comfort in aniridia. They reported no success in cases of nystagmus fitted in their clinic. Bier suggested that contact lenses may prove helpful in nystagmus and that cosmetic lenses may help fixation in aniridia. No details of cases, nor special techniques were described in the latter two reports. Girard ' suggested that in patients with aniridia, acuity may be improved and nystagmus reduced in amplitude and frequency with contact lenses with a painted iris. In an adult patient he presented, nystagmus showed remission but visual acu1
2
3
4 5
From the Department of Ophthalmology, Washington University School of Medicine. This research was supported in part by Career Development Award 5-K3-NB 15138, U S P H S Grants N B 03639 and N B 06525 of the National Institute of Neurological Diseases and Blindness, National Institutes of Health.
M.D.
ity was not improved. Reid reported fitting contact lenses with an opaque brown corneal portion with a 2.5 mm clear pupillary zone to an adult with aniridia without nystagmus, and achieving an excellent result. Pincus reported some visual improvement in five adult aniridic patients fitted with contact lenses having opaque peripheral corneal zones. Duke-Elder presented an excellent review of many aspects of aniridia. 6
7
8
In a number of adult nystagmus cases one of us ( J M E ) has seen, including two patients with partial albinism, contact lenses did not seem particularly advantageous, although the patients generally seemed pleased with their corrections. They provided equivalent optical and cosmetic improvements expected in the correction of other high refractive errors. It might be added that these patients presented no special problems during fitting. CASE REPORT
A white male infant was first seen at the age of 8 weeks with marked photophobia and extremely "large pupils." The child was the first-born; the mother had had a fullterm normal pregnancy complicated only by a breech birth. Birth weight was six pounds nine ounces. No viral illness occurred during pregnancy nor was there any unusual drug intake. There was no family history of any ocular abnormalities on either the maternal or paternal sides. Examination revealed a normal-appearing infant with photophobia and tearing. Bilateral aniridia was noted. The corneas were clear and normal-sized. No nystagmus was present and ocular motility in dim illumination appeared normal. Examination under halothane general anesthesia revealed corneal diameters of 10.7 mm in both eyes. The corneas were clear. Retinoscopy indicated a —3.00 diopter sphere refractive error in both eyes. Under
334
AMERICAN JOURNAL OF OPHTHALMOLOGY
the Zeiss microscope, the lenses appeared normal in size and position. However, a well-developed tunica vasculosa lentis was present with blood-flow visible through the vessels. Intraocular pressures were right eye, 12 mm Hg and left eye, 16 mm Hg. Gonioscopy revealed a nubbin of iris root which in 90% of the angle did not encumber the trabecular meshwork. A few pectinate ligaments were visible. Attempts at tonography were unsuccessful due to the narrow fissure and Bell's phenomenon. Examination of the fundi showed normal optic nerves. The macular areas were avascular but otherwise normal. A routine physical examination showed normal findings. X-rays of the abdomen revealed no evidence of Wilm's tumor. 8
When the child was four months old, a variable jerk-type horizontal nystagmus developed. Within a short period of time, the nystagmus became quite marked. It was decided to use scleral-type contact lenses with artificial irides in an effort to control the amount of light entering the eye and the quality of the retinal image. He was examined again under general anesthesia at the age of 5yi months. The intraocular pressures were normal, the corneal diameters were 10.7 mm and the tunica vasculosa lentis had almost disappeared. The fundi appeared unchanged. Special small injection molding shells were constructed to correspond to the child's lid aperture ( 19 mm horizontal, 12 mm vertical with lids separated). The molding for the lenses was done with the child under general anesthesia. A serious problem was encountered with the tendency of the eyes to turn up during anesthesia. Excellent molds were obtained of the cornea and the lower and horizontal aspects of the infant's eyes. Compensation was made for the limited superior scleral area in the following manner: The mold was spun and, in forming the lens, the contour of the lower part of the eye was used for the upper half of the lens. This approach is indicated only when the haptic zones are essentially nonastigmatic. The contact lens we used resembled the
A U G U S T , 1968
early Obrig "Lacrilens," featuring two V slits, one above and one below the cornea. The V-slits allow tear and gaseous exchange. This early type of scleral vented lens is no longer commonly used because of inherent structural weakness, but we felt that this property was advantageous for this infant, to limit added pressure on the eye. Further, we wanted to interfere minimally with natural ocular growth and development. An 8.15-mm radius inner corneal curve was used and corneoscleral limbus clearance was provided. The two V-slits allow the haptic portion to be divided (for fitting purposes) into six segments—three on each side. A satisfactory fit was achieved after about eight visits. These lenses probably will allow modification for at least a few years, judging by the ease of adjustment. W e had occasional problems, one being that the conjunctiva tended to roll up over the edges of the lenses. On one occasion edema and conjunctival hemorrhage occurred when the lens was removed with a suction cup, and the conjunctiva trapped between the suction cup and the lens. The parents have since gained skill at insertion and removal of the lenses, and this has not occurred during the past six months. The lenses were originally fitted without the painted irides, to allow evaluation of the corneal fit. They were then replicated with the laminated painted iris. Only limited modification was required for the final lenses. Scleral lenses were employed because it was necessary for the painted irides to cover the entire cornea. A 2.5-mm clear optical zone was located in the center of each painted iris (fig. 1 ) , and the myopia was corrected. After a period of six months' wear for about eight hours a day, there was no measurable change in refraction or evidence of ocular irritation. The infant, now 20 months old, wears the lenses with comfort, and is considerably more alert when wearing them. Most important is the fact that the nystagmus, which first appeared at about the time contact lens fitting was initiated (age 5j4 months), has shown marked remission. The fitting required al-
V O L . 66, N O . 2
REMISSION OF NYSTAGMUS
335 SUMMARY
Congenital bilateral aniridia was described in a 20-month-old white male infant with no family history of ocular abnormalities. The child was fitted with vented scleral contact lenses which he has tolerated well. His photophobia has been eliminated, there has been marked remission of his developing nystagmus, and he appears to have useful vision for his activities. It is hoped that prompt treatment will improve the visual prognosis in such cases. Fig. 1 (Enoch and Windsor). This is one of the lenses fitted to the infant with congenital aniridia. The clear pupillary aperture was 2.5 mm.
most seven months—including the time spent in instructing the parents how to insert and remove the contact lenses. Nystagmus is still present to some degree when the lenses are removed, when the child is sleepy, and on extreme ocular versions. However, during normal contact lens wear, few and only fine nystagmoid movements are observed. An alternating esotropia appears to be developing which may require treatment in the future. It is important to learn why any case of nystagmus shows remission, and the influence of remission on vision. In this patient, optical techniques resulted in reduced aberrations, reduced photophobia, increased depth of focus, and in an improved refractive correction. Early treatment proved to be clearly beneficial.
ADDENDUM
A parallel study has recently been published : Harmann, J. S . and Kovermann, J. J. : Prophylaxis of amblyopia in aniridia : The role of pinhole contact lenses. J. Pediat. Ophth. 5:48, 1968. REFERENCES 1. Grosvenor, T . : Contact Lenses Theory and Practice. Chicago, Profession Press, 1963, p. 340. 2. Iliff, C. E. and Naquin, H . A. : Indications. Tr. Am. Acad. Ophth. Otolaryng. 66:303, 1962. 3. Bier, N. : Contact Lens, Routine and Practice. London, Butterworth, 1957, ed. 2, p. 124. 4. Girard, L. : Corneal Contact Lenses. St. Louis, Mosby, 1964, p. 117. 5. Girard, L., Camp, R N., and Lamb, V. R , Richardson, C. B. and Soper, J. W . : Iris corneal contact lens. Am. J. Ophth. 52:264, 1961. 6. Reid, A. M . : Case of congenital aniridia fitted with pigmented contact glasses. Tr. Ophth. Soc. U.K. 58 (pt. 1) :434, 1938. 7. Pincus, M. H . : Aniridia Congenita. Arch. Ophth. 39:60,1948. 8. Duke-Elder, S. : System of Ophthalmology, Vol. 3, Normal and Abnormal Development, pt. 2. S t Louis, Mosby, 1963, p. 566. 9. DiGeorge, A . M . and Harley, R. D. : The association of aniridia, Wilm's tumor, and genital abnormalities. Tr. Am. Ophth. Soc. 63:64, 1965.
Congenital aniridia has a poor visual prognosis. Glaucoma, dislocated lenses, strabismus, nystagmus, and other ocular anomalies interfere with the development of useful vision. Even if glaucoma and retinal abnormalities do not develop, uncontrolled retinal illumination, increased ocular aberrations, and decreased depth of focus due to absence of the pupillary mechanism, in some manner cause the development of nystagmus and subsequent subnormal vision. In the following case the fitting of vented scleral contact lenses with artificial irides not only eliminated the patient's photophobia, but appeared to reduce markedly his nystagmus. This case will be followed to ascertain whether these and subsequent lenses will serve to inhibit further the development of nystagmus. Grosvenor has summarized suggestions in the literature that optically-induced nystagmus shows remission following fitting of contact lenses. None of the patients reported were infants and none had aniridia. Iliff and Naquin indicated that contact lenses failed to improve vision, but gave visual comfort in aniridia. They reported no success in cases of nystagmus fitted in their clinic. Bier suggested that contact lenses may prove helpful in nystagmus and that cosmetic lenses may help fixation in aniridia. No details of cases, nor special techniques were described in the latter two reports. Girard ' suggested that in patients with aniridia, acuity may be improved and nystagmus reduced in amplitude and frequency with contact lenses with a painted iris. In an adult patient he presented, nystagmus showed remission but visual acu1
2
3
4 5
From the Department of Ophthalmology, Washington University School of Medicine. This research was supported in part by Career Development Award 5-K3-NB 15138, U S P H S Grants N B 03639 and N B 06525 of the National Institute of Neurological Diseases and Blindness, National Institutes of Health.
M.D.
ity was not improved. Reid reported fitting contact lenses with an opaque brown corneal portion with a 2.5 mm clear pupillary zone to an adult with aniridia without nystagmus, and achieving an excellent result. Pincus reported some visual improvement in five adult aniridic patients fitted with contact lenses having opaque peripheral corneal zones. Duke-Elder presented an excellent review of many aspects of aniridia. 6
7
8
In a number of adult nystagmus cases one of us ( J M E ) has seen, including two patients with partial albinism, contact lenses did not seem particularly advantageous, although the patients generally seemed pleased with their corrections. They provided equivalent optical and cosmetic improvements expected in the correction of other high refractive errors. It might be added that these patients presented no special problems during fitting. CASE REPORT
A white male infant was first seen at the age of 8 weeks with marked photophobia and extremely "large pupils." The child was the first-born; the mother had had a fullterm normal pregnancy complicated only by a breech birth. Birth weight was six pounds nine ounces. No viral illness occurred during pregnancy nor was there any unusual drug intake. There was no family history of any ocular abnormalities on either the maternal or paternal sides. Examination revealed a normal-appearing infant with photophobia and tearing. Bilateral aniridia was noted. The corneas were clear and normal-sized. No nystagmus was present and ocular motility in dim illumination appeared normal. Examination under halothane general anesthesia revealed corneal diameters of 10.7 mm in both eyes. The corneas were clear. Retinoscopy indicated a —3.00 diopter sphere refractive error in both eyes. Under
334
AMERICAN JOURNAL OF OPHTHALMOLOGY
the Zeiss microscope, the lenses appeared normal in size and position. However, a well-developed tunica vasculosa lentis was present with blood-flow visible through the vessels. Intraocular pressures were right eye, 12 mm Hg and left eye, 16 mm Hg. Gonioscopy revealed a nubbin of iris root which in 90% of the angle did not encumber the trabecular meshwork. A few pectinate ligaments were visible. Attempts at tonography were unsuccessful due to the narrow fissure and Bell's phenomenon. Examination of the fundi showed normal optic nerves. The macular areas were avascular but otherwise normal. A routine physical examination showed normal findings. X-rays of the abdomen revealed no evidence of Wilm's tumor. 8
When the child was four months old, a variable jerk-type horizontal nystagmus developed. Within a short period of time, the nystagmus became quite marked. It was decided to use scleral-type contact lenses with artificial irides in an effort to control the amount of light entering the eye and the quality of the retinal image. He was examined again under general anesthesia at the age of 5yi months. The intraocular pressures were normal, the corneal diameters were 10.7 mm and the tunica vasculosa lentis had almost disappeared. The fundi appeared unchanged. Special small injection molding shells were constructed to correspond to the child's lid aperture ( 19 mm horizontal, 12 mm vertical with lids separated). The molding for the lenses was done with the child under general anesthesia. A serious problem was encountered with the tendency of the eyes to turn up during anesthesia. Excellent molds were obtained of the cornea and the lower and horizontal aspects of the infant's eyes. Compensation was made for the limited superior scleral area in the following manner: The mold was spun and, in forming the lens, the contour of the lower part of the eye was used for the upper half of the lens. This approach is indicated only when the haptic zones are essentially nonastigmatic. The contact lens we used resembled the
A U G U S T , 1968
early Obrig "Lacrilens," featuring two V slits, one above and one below the cornea. The V-slits allow tear and gaseous exchange. This early type of scleral vented lens is no longer commonly used because of inherent structural weakness, but we felt that this property was advantageous for this infant, to limit added pressure on the eye. Further, we wanted to interfere minimally with natural ocular growth and development. An 8.15-mm radius inner corneal curve was used and corneoscleral limbus clearance was provided. The two V-slits allow the haptic portion to be divided (for fitting purposes) into six segments—three on each side. A satisfactory fit was achieved after about eight visits. These lenses probably will allow modification for at least a few years, judging by the ease of adjustment. W e had occasional problems, one being that the conjunctiva tended to roll up over the edges of the lenses. On one occasion edema and conjunctival hemorrhage occurred when the lens was removed with a suction cup, and the conjunctiva trapped between the suction cup and the lens. The parents have since gained skill at insertion and removal of the lenses, and this has not occurred during the past six months. The lenses were originally fitted without the painted irides, to allow evaluation of the corneal fit. They were then replicated with the laminated painted iris. Only limited modification was required for the final lenses. Scleral lenses were employed because it was necessary for the painted irides to cover the entire cornea. A 2.5-mm clear optical zone was located in the center of each painted iris (fig. 1 ) , and the myopia was corrected. After a period of six months' wear for about eight hours a day, there was no measurable change in refraction or evidence of ocular irritation. The infant, now 20 months old, wears the lenses with comfort, and is considerably more alert when wearing them. Most important is the fact that the nystagmus, which first appeared at about the time contact lens fitting was initiated (age 5j4 months), has shown marked remission. The fitting required al-
V O L . 66, N O . 2
REMISSION OF NYSTAGMUS
335 SUMMARY
Congenital bilateral aniridia was described in a 20-month-old white male infant with no family history of ocular abnormalities. The child was fitted with vented scleral contact lenses which he has tolerated well. His photophobia has been eliminated, there has been marked remission of his developing nystagmus, and he appears to have useful vision for his activities. It is hoped that prompt treatment will improve the visual prognosis in such cases. Fig. 1 (Enoch and Windsor). This is one of the lenses fitted to the infant with congenital aniridia. The clear pupillary aperture was 2.5 mm.
most seven months—including the time spent in instructing the parents how to insert and remove the contact lenses. Nystagmus is still present to some degree when the lenses are removed, when the child is sleepy, and on extreme ocular versions. However, during normal contact lens wear, few and only fine nystagmoid movements are observed. An alternating esotropia appears to be developing which may require treatment in the future. It is important to learn why any case of nystagmus shows remission, and the influence of remission on vision. In this patient, optical techniques resulted in reduced aberrations, reduced photophobia, increased depth of focus, and in an improved refractive correction. Early treatment proved to be clearly beneficial.
ADDENDUM
A parallel study has recently been published : Harmann, J. S . and Kovermann, J. J. : Prophylaxis of amblyopia in aniridia : The role of pinhole contact lenses. J. Pediat. Ophth. 5:48, 1968. REFERENCES 1. Grosvenor, T . : Contact Lenses Theory and Practice. Chicago, Profession Press, 1963, p. 340. 2. Iliff, C. E. and Naquin, H . A. : Indications. Tr. Am. Acad. Ophth. Otolaryng. 66:303, 1962. 3. Bier, N. : Contact Lens, Routine and Practice. London, Butterworth, 1957, ed. 2, p. 124. 4. Girard, L. : Corneal Contact Lenses. St. Louis, Mosby, 1964, p. 117. 5. Girard, L., Camp, R N., and Lamb, V. R , Richardson, C. B. and Soper, J. W . : Iris corneal contact lens. Am. J. Ophth. 52:264, 1961. 6. Reid, A. M . : Case of congenital aniridia fitted with pigmented contact glasses. Tr. Ophth. Soc. U.K. 58 (pt. 1) :434, 1938. 7. Pincus, M. H . : Aniridia Congenita. Arch. Ophth. 39:60,1948. 8. Duke-Elder, S. : System of Ophthalmology, Vol. 3, Normal and Abnormal Development, pt. 2. S t Louis, Mosby, 1963, p. 566. 9. DiGeorge, A . M . and Harley, R. D. : The association of aniridia, Wilm's tumor, and genital abnormalities. Tr. Am. Ophth. Soc. 63:64, 1965.